Creutzfeldt-Jakob Disease, all types (Transmissible Spongiform Encephalopathy)

Reporting Obligations

Individuals with suspect or confirmed cases must be reported to the Thunder Bay District Health Unit by the next working day by fax, phone or mail.

  • Fax: (807) 625-4822
  • Phone: 625-8318 or toll-free at 1-888-294—6630, ext. 8318
  • Mail: 999 Balmoral Street, Thunder Bay, ON P7B 6E7

 

Epidemiology

Aetiologic Agent:

Aetiology is not clear, but the infectious agents are thought to be unique proteins called prions which replicate by an unknown mechanism.

Clinical Presentation:

TSEs or prion diseases, constitute a group of rare, rapidly progressive, universally fatal neuro-degenerative syndromes that are characterized by neuronal degeneration, spongiform change, gliosis and accumulation of an abnormal protease-resistant amyloid protein or scrapie prion protein distributed diffusely throughout the brain and sometimes also in discrete plaques.

Clinical presentation manifests as a dementing syndrome with symptoms of confusion, behavioural and cognitive abnormalities, progressive dementia and variable ataxia and dysarthria.

Modes of Transmission:

The mode of transmission for sporadic disease is unknown; some cases of CJD have occurred iatrogenically; some types of TSEs are genetic; and vCJD is believed to be transmitted from BSE- infected cattle.

Incubation Period:

Route of exposure influences incubation period; 15 – 120 months with direct CNS exposure.

Period of Communicability:

Transmissibility and period of communicability vary with disease, tissue involved and stage of disease. CNS and other tissues are infectious throughout symptomatic illness; lymphoid and other organs are probably infectious before signs of illness appear. There is evidence that blood may be infective in some forms of experimental prion disease.

 

Risk Factors/Susceptibility

Risk factors are not completely known given that modes of transmission are not fully understood. Exposure to TSE is thought to be possible through blood transfusion/transplants of infected individuals; through the use of medicinal products which were made with infected bovine materials; or through consumption of beef products that have been contaminated with the bovine form of spongiform encephalopathy (BSE). Refer to "WHO Guidelines on Tissue Infectivity Distribution in Transmissible Spongiform Encephalopathies" as listed below under additional resources.

 

Diagnosis & Laboratory Testing

Confirmed case:  Neuropathologically / immunocytochemically confirmed: confirmation of protease-resistant prion protein (immunocytochemistry or Western Blot).

Probable case: 

• Rapidly progressive dementia AND

• At least two additional neurological manifestations AND

• Typical electroencephalography (EEG): generalized bilateral or unilateral triphasic periodic complexes at approximately one per second, lasting continuously for at least 10 seconds OR

• Suspect sporadic CJD AND

• Positive assay for 14-3-3 in cerebrospinal uid (CSF)

Suspect case:

• Rapidly progressive dementia AND

• At least two additional neurological manifestations AND

• Duration of illness less than 2 years

Laboratory Confirmation:

The following will constitute a con rmed case of Sporadic Creutzfeldt-Jakob Disease:

• Neuropathological con rmation of protease-resistant prion protein (immunocytochemistry in situ or via PET blot; or Western Blot).

Clinical Evidence:

Additional neurological manifestations include:

• Myoclonus

• Visual or cerebellar disturbances such as ataxia

• Pyramidal or extrapyramidal features

• Akinetic mutism

Note: A clinical consultation is necessary for diagnosis.

Testing Information & Requisition

 

Treatment & Case Management

There is no specific treatment available. Infected persons as well as their family members should be excluded from donating blood, organs and other body tissues. Persons diagnosed with this infection who have made donations as listed above are to be reported to Canadian blood services (CBS) to enable look back and recall procedures.

 

Patient Information

Patient Fact Sheet

 

References

  1. Ministry of Health and Long Term Care, Infectious Diseases Protocol, 2016. Appendix A (2009) and Appendix B (2009)
  2. Heymann, D.L., Control of Communicable Disease Manual (20th Ed.), Washington, American Public Health Association, 2015.

 

Additional Resources

1. Centers for Disease Control and Prevention. "CJD (Creutzfeldt-Jakob Disease, Classic)."

2. Centers for Disease Control and Prevention. "vCJD (Variant Creutzfeldt-Jakob Disease)."

3. World Health Organization. "WHO Guidelines on Tissue Infectivity Distribution in Transmissible Spongiform Encephalopathies."

Last Updated: 26/10/2017